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Thorac Cardiovasc Surg 2000; 48(4): 203-208
DOI: 10.1055/s-2000-6893
Original Cardiovascular
ORIGINALARBEIT
© Georg Thieme Verlag Stuttgart · New York

Morphological Aspects of Ebstein's Anomaly in Adults

C. Frescura, A. Angelini, L. Daliento, G. Thiene
  • Departments of Pathology and Cardiology, University of Padua Medical School, Padua, Italy
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Publication History

Publication Date:
31 December 2000 (online)

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The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium (“atrialized portion”), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.

Key words:

- Congenital heart disease - Sudden death - Tricuspidvalve dysplasia

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Gaetano Thiene

Istituto di Anatomia Patologica

Via A. Gabelli 61

35121 Padova

Italy

Phone: +++ 39 049 8272283

Fax: +++39 049 8272284

Email: cardiopath@ux1.unipd.it

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